Article (Scientific journals)
Unusual Cardiac Manifestations of a Pheochromocytoma in a Girl.
D'Angelo, Lisa; Parent, Anne-Simone; Derwael, Céline et al.
2023In Pediatric Reports, 15 (1), p. 237-244
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Keywords :
aortic root dilation; left ventricular hypertrophy; pheochromocytoma; prolonged QT interval; von Hippel-Lindau gene mutation
Abstract :
[en] We report the case of an 11-year-old girl who complained about severe asthenia, orthostatic dizziness and abdominal pain for 4 weeks. The primary investigation concluded on febrile urinary tract infection treated by antibiotics. Symptom persistence prompted cardiological and endocrinological investigations. A fluctuation in blood pressure, long QT interval, dilation of the aortic root and left ventricular hypertrophy were documented. Elevated levels of urinary catecholamines together with the presence of a right-sided adrenal mass shown via abdominal ultrasound and magnetic resonance imaging were highly suggestive of a pheochromocytoma. This was confirmed by through iodine-123-metaiodobenzylguathdine ([(123)I]-mIBG) scintigraphy. Genetic analysis allowed for the exclusion of pathogenic mutations in genes implicated in hereditary paragangliomas and pheochromocytomas but showed a rare somatic mutation in exon 3 of the von Hippel-Lindau gene. The patient was treated with a β-blocker and calcium channel antagonist and underwent laparoscopic right-sided adrenalectomy. Cardiac manifestations resolved soon after surgery indicating that they were secondary to the pheochromocytoma. After 5 years of follow-up, the patient remains asymptomatic without any sign of tumor recurrence. The presence of aortic root dilation, a prolonged QT-interval and left ventricular hypertrophy may be early cardiac manifestations of a pheochromocytoma in a child and should prompt this diagnosis to be evoked.
Disciplines :
Pediatrics
Cardiovascular & respiratory systems
Author, co-author :
D'Angelo, Lisa ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
Parent, Anne-Simone ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
Derwael, Céline  ;  Centre Hospitalier Universitaire de Liège - CHU > > Service médical de médecine nucléaire et imagerie onco
Hustinx, Roland  ;  Centre Hospitalier Universitaire de Liège - CHU > > Service médical de médecine nucléaire et imagerie onco
Seghaye, Marie-Christine  ;  Centre Hospitalier Universitaire de Liège - CHU > > Service de pédiatrie
Language :
English
Title :
Unusual Cardiac Manifestations of a Pheochromocytoma in a Girl.
Publication date :
17 March 2023
Journal title :
Pediatric Reports
ISSN :
2036-749X
eISSN :
2036-7503
Publisher :
PAGEPress Publications, Pavia, It
Volume :
15
Issue :
1
Pages :
237-244
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 08 June 2023

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